Liu TZ, Zheng YJ, Zhang ZW, Li SS, Chen JT, Peng AH, Huang RW. Chidamide based combination regimen for treatment of monomorphic epitheliotropic intestinal T cell lymphoma following radical operation: Two case reports. World J Clin Cases 2020; 8(7): 1278-1286 [PMID: 32337203 DOI: 10.12998/wjcc.v8.i7.1278]
Corresponding Author of This Article
Ren-Wei Huang, MD, Professor, Chief Doctor, Department of Hematology, The Sixth Affiliated Hospital of Sun Yat-Sen University, 26 Yuancun Erheng Road, Guangzhou 510655, Guangdong Province, China. huangrw56@163.com
Research Domain of This Article
Medicine, Research & Experimental
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Apr 6, 2020; 8(7): 1278-1286 Published online Apr 6, 2020. doi: 10.12998/wjcc.v8.i7.1278
Chidamide based combination regimen for treatment of monomorphic epitheliotropic intestinal T cell lymphoma following radical operation: Two case reports
Ting-Zhi Liu, Yi-Jia Zheng, Jiao-Ting Chen, Ai-Hua Peng, Ren-Wei Huang, Department of Hematology, The Sixth Affiliated Hospital of Sun Yat-Sen University, Guangzhou 510655, Guangdong Province, China
Zhan-Wen Zhang, Department of Nuclear Medicine, The Sixth Affiliated Hospital of Sun Yat-Sen University, Guangzhou 510655, Guangdong Province, China
Shan-Shan Li, Department of Oncology, The Sixth Affiliated Hospital of Sun Yat-Sen University, Guangzhou 510655, Guangdong Province, China
Author contributions: Liu TZ acquired the data and drafted the article; Zheng YJ, who was the resident physician of case two, analyzed the data and drafted the article; Zhang ZW analyzed and interpreted the imaging findings; Li SS helped revise the article; Chen JT, who was the resident physician of case one, analyzed the data; Peng AH and Huang RW, who were the chief physicians of cases one and two, revised the manuscript for important intellectual content. All authors issued final approval for the version to be submitted.
Informed consent statement: We obtained written informed consent from patients before treatment administering. When we did this retrospective analysis, we obtained oral informed consent from their relatives for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Ren-Wei Huang, MD, Professor, Chief Doctor, Department of Hematology, The Sixth Affiliated Hospital of Sun Yat-Sen University, 26 Yuancun Erheng Road, Guangzhou 510655, Guangdong Province, China. huangrw56@163.com
Received: December 24, 2019 Peer-review started: December 24, 2019 First decision: March 5, 2020 Revised: March 18, 2020 Accepted: March 22, 2020 Article in press: March 22, 2020 Published online: April 6, 2020 Processing time: 103 Days and 9.3 Hours
Abstract
BACKGROUND
Monomorphic epitheliotropic intestinal T cell lymphoma (MEITL) is a rare extra-nodal T-cell lymphoma that has uniformly aggressive features with a poor prognosis. No standardized treatment protocols have been established. Previous experience has demonstrated favorable outcomes with combination chemotherapy followed by autologous hematopoietic stem cell transplant. However, many patients are unable to tolerate the toxicities. Chidamide is a new histone deacetylase inhibitor that has shown preferential efficacy in mature T-cell lymphoma.
CASE SUMMARY
We herein present two cases of MEITL who were both intermediate risk according to enteropathy-associated T cell lymphoma prognostic index. Case one was a 61-year-old man. He complained of upper abdominal pain and intermittent black stool for 2 mo. Imaging examination revealed that the intestinal wall was thickened. He received a partial excision of the small intestine. A chidamide-based combination regimen was given postoperatively. Eleven months later, he presented with recurrence in the bilateral lungs. He passed away 15 mo after his diagnosis. Case two was a 35-year-old woman who complained of abdominal distention for 1 mo. Positron emission tomography/computed tomography demonstrated wall thickening of the small intestine and upper sigmoid colon. Colon perforation and septic shock occurred on the fourth day of her admission. She was treated by sigmoid colostomy. Chidamide-based combination therapy was then provided. She was recurrence-free for 6 mo until lesions were found in the bilateral brain and lived for 17 mo since her diagnosis. Compared to historical data, chidamide seems to improve the prognosis of MEITL slightly.
CONCLUSION
MEITL is a type of aggressive lymphoma. Chidamide is a new promising approach for the treatment of MEITL.
Core tip: Monomorphic epitheliotropic intestinal T cell lymphoma (MEITL) is a rare and aggressive disease with a poor prognosis. No standardized treatment has been established yet. We report two cases of patients with MEITL who were treated by radical resection followed by chidamide combined with chemotherapy and review the literature focusing on histopathological characteristics, etiology, diagnosis, treatment strategies, and outcomes of MEITL. This report will improve our understanding of MEITL.
